Brain, Vol 121, Issue 4 561-579, Copyright © 1998 by Oxford University Press
JD Schmahmann and JC Sherman
Anatomical, physiological and functional neuroimaging studies suggest that
the cerebellum participates in the organization of higher order function,
but there are very few descriptions of clinically relevant cases that
address this possibility. We performed neurological examinations, bedside
mental state tests, neuropsychological studies and anatomical neuroimaging
on 20 patients with diseases confined to the cerebellum, and evaluated the
nature and severity of the changes in neurological and mental function.
Behavioural changes were clinically prominent in patients with lesions
involving the posterior lobe of the cerebellum and the vermis, and in some
cases they were the most noticeable aspects of the presentation. These
changes were characterized by: impairment of executive functions such as
planning, set-shifting, verbal fluency, abstract reasoning and working
memory; difficulties with spatial cognition including visual-spatial
organization and memory; personality change with blunting of affect or
disinhibited and inappropriate behaviour; and language deficits including
agrammatism and dysprosodia. Lesions of the anterior lobe of the cerebellum
produced only minor changes in executive and visual- spatial functions. We
have called this newly defined clinical entity the 'cerebellar cognitive
affective syndrome'. The constellation of deficits is suggestive of
disruption of the cerebellar modulation of neural circuits that link
prefrontal, posterior parietal, superior temporal and limbic cortices with
the cerebellum.
ARTICLES
The cerebellar cognitive affective syndrome
Department of Neurology, Massachusetts General Hospital, Boston 02114, USA.
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